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Anomalous origin of the right pulmonary artery from the ascending aorta accompanied by absent pulmonary valve syndrome and right-sided aortic arch: a rare case in adult congenital heart disease

Published online by Cambridge University Press:  01 July 2014

Zahra Khajali ,
Ali Mohammadzadeh  and
Marzieh Khayatzadeh
Zahra Khajali
Affiliation:
Fellowship of Echocardiography, Echocardiography Department, Rajaie Cardiovascular, Medical and Research Center, Iran University of Medical Sciences, Tehran, IR Iran
Ali Mohammadzadeh
Affiliation:
Fellowship of Echocardiography, Echocardiography Department, Rajaie Cardiovascular, Medical and Research Center, Iran University of Medical Sciences, Tehran, IR Iran
Marzieh Khayatzadeh*
Affiliation:
Fellowship of Echocardiography, Echocardiography Department, Rajaie Cardiovascular, Medical and Research Center, Iran University of Medical Sciences, Tehran, IR Iran
*
Correspondence to: Dr M. Khayatzadeh, MD; Fellowship of Echocardiography, Echocardiography Department, Rajaie Cardiovascular, Medical and Research Center, Iran University of Medical Sciences, Tehran 1996911151, IR Iran. Tel: +989123276710; Fax: +982122055594; E-mail: marzi_khayat@yahoo.com
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Abstract

We present a rare congenital heart disease in a 20-year-old man with anomalous origin of the right pulmonary artery from the ascending aorta, accompanied by absent pulmonary valve syndrome, and a right-sided aortic arch suspected initially in transthoracic echocardiography and subsequently confirmed by cardiac catheterisation and computed tomography angiography.

Keywords

Anomalous origin of right pulmonary arteryabsent pulmonary valve syndromeright-sided aortic arch
Type
Brief Reports
Information
Cardiology in the Young , Volume 25 , Issue 4 , April 2015 , pp. 794 - 796
Copyright
© Cambridge University Press 2014 

The anomalous origin of one pulmonary artery branch from the aorta and the other branch from the right ventricle via the main pulmonary artery is a very rare congenital anomaly and was first described by Fraentzel.Reference Fraentzel 1 Other cardiac anomalies such as tetralogy of Fallot, patent ductus arteriosus, and aortopulmonary window, which are defined as a congenital syndrome, may be associated with this disease.

Case report

A 20-year-old man was referred to our hospital for dyspnoea of a duration of 10 years. Physical examination revealed mild central and peripheral cyanosis, clubbing, holosystolic ejection and diastolic murmur along the left sternal border, and narrow S1 and absent S2 sounds. The patient had resting O2 saturation in room air of 85%, blood pressure of 110/60 mm Hg, and heart rate of 80 beats/minute. Paraclinical data showed haemoglobin concentration of 16.3 g/dl and haematocrit of 49.2%. Electrocardiography revealed sinus rhythm, right-axis deviation, and incomplete right bundle branch block. Chest X-ray illustrated cardiomegaly, increased perfusion to the peripheral segments in the left lung, and evidence of pruning decreased pulmonary vasculature at the distal one-third of the right lung. Transthoracic echocardiography demonstrated overriding of the aorta with a subaortic ventricular septal defect (2 cm), hypertrophied right ventricle, poorly formed pulmonary valve leaflets in favour of the absent pulmonary valve with annular stenosis (peak pressure gradient of about 80 mm Hg), severe pulmonary insufficiency, and non-confluent pulmonary branches, with the right pulmonary artery arising from the left side of the ascending aorta, and the left pulmonary artery arising from the right ventricle (Fig 1).

Figure 1 Overriding of aorta with large subaortic ventricular septal defect (arrow) in long axis view, ( a ) absent pulmonary valve and left pulmonary artery (arrow) which is the sole artery arising from the right ventricle in short axis view in transthoracic echocardiography, ( b ) right pulmonary artery arising from ascending aorta in right parasternal view in transthoracic echocardiography (arrow), ( c ) • location of main pulmonary artery.

Haemodynamic assessments at cardiac catheterisation revealed aortic and pulmonary saturation of 75 and 76%, respectively, systemic right pulmonary artery pressure, pulmonary vascular resistance of 13 wood units, and left lung pulmonary vascular resistance of about 8 wood units (pulmonary artery pressure of 80 mm Hg). Contrast injection of the right ventricle exhibited the opacification of the main and left pulmonary arteries, and aortic root injection showed an anomalous origin of the right pulmonary artery from the left side of the ascending aorta. Computed tomography angiography revealed that the right pulmonary artery originated from the posterior left lateral side of the ascending aorta, which descended posteriorly towards the right hilum – anomalous origin of the pulmonary artery from the ascending aorta. The right pulmonary artery was measured to be about 25 mm, showing the peripheral sub-segmental branches tapering, with pruning at the periphery of the right lung, in favour of supra-systemic pulmonary artery pressure. The left pulmonary artery was the sole artery originating from the right ventricular outflow tract, with the engorgement of its trunk (30 mm) and intra-parenchymal branches, particularly the left descending pulmonary artery (Fig 2).

Figure 2 Anomalous origin of right pulmonary artery from left posterior side of ascending aorta. Aortic root injection at cardiac catheterization, ( a ) axial view of aorta and pulmonary artery in computed tomography (CT) angiography, showed left pulmonary artery is the sole artery originating from main pulmonary artery, ( b ) reconstructed coronal view of aorta and pulmonary artery in CT angiography revealed anomalous origin of right pulmonary artery from left posterolateral side of ascending aorta (arrow) which descend posteriorly towards right hillum, ( c ) *location of right pulmonary artery; LPA=left pulmonary artery; MPA=main pulmonary artery; RPA=right pulmonary artery.

Given the diagnosis of the anomalous origin of the right pulmonary artery from the aorta, resulting in systemic pressure and increased pulmonary vascular resistance in the right lung, accompanied by acceptable pulmonary vascular resistance in the left lung, the surgical plan was ventricular septal defect closure with pulmonary valve replacement and leaving the right pulmonary artery unoperated. However, the patient refused surgery.

Discussion

The anomalous origin of one pulmonary artery from the ascending aorta accounts for 0.12% of all congenital heart diseases, and absent pulmonary valve syndrome is responsible for 3–6% of all cases of tetralogy of Fallot;Reference Erdem, Aydemir and Demir 2 , Reference Aly, Red, Jiwani and Desai 3 the combination of both anomalies is, however, very rare. The anomalous origin of the right pulmonary artery from the ascending aorta is more common than is the anomalous origin of the left pulmonary artery; however, the latter is more commonly associated with tetralogy of Fallot and right-sided aortic arch.Reference Patel, Zakir and Sethi 4 , Reference Dwived, Vijay, Chandra and Saran 5 Absent pulmonary valve syndrome has a similar combination of problems, which are seen in tetralogy of Fallot: ventricular septal defect; overriding of the aorta; and narrow pulmonary valve (poorly formed), which results in severe pulmonary incompetency as well as stenosis. Absent pulmonary valve syndrome is more commonly seen in combination with the anomalous origin of the left pulmonary artery from the ascending aorta, based on previous reviews of the literature.Reference Aly, Red, Jiwani and Desai 3 , Reference Presbitero, Pedretti and Orzan 6

In patients with the anomalous origin of one pulmonary artery from the ascending aorta, accompanied by absent pulmonary valve syndrome, after birth because of low pulmonary vascular resistance, both the lungs are exposed to high volume and pressure load. The lung that receives the systemic artery from the aorta has systemic pressure, and the other lung is the sole one receiving the whole right ventricular flow. Cardiac catheterisation is regarded as a suitable guide for decision making in that it confers an assessment of the pulmonary vascular resistance of both the lungs. Overall, pulmonary vascular resistance of up to the value of 8 wood units is usually considered for surgery. Early diagnosis and surgical interventions at infancy are life-saving, because overload causes pulmonary vascular disease and increases resistance. Indeed, only a few patients survive until adulthood. For our patient, we opted for surgical ventricular septal defect closure and pulmonary valve replacement, given the pulmonary vascular resistance of the left lung, but the patient refused surgery.

To the best of our knowledge, in the existing literature, this is the first report on a complex congenital syndrome containing the anomalous origin of the right pulmonary artery from the left side of the ascending aorta, accompanied by absent pulmonary valve syndrome and a right-sided aortic arch.

Acknowledgement

None.

Financial Support

This research received no specific grants from any funding agency, commercial, or not-for-profit sectors.

Conflicts of Interest

None.

References

1. Fraentzel, O. Ein Fall von abnormer Communication der aorta mit der Arteria pulmonalis. Virchows Arch Pathol Ana 1868; 43: 420–426.CrossRefGoogle Scholar
2. Erdem, A, Aydemir, NA, Demir, H, et al. Anomalous origin of one pulmonary artery branch from the ascending aorta: experience of our center. Arch Turk Soc Cardiol 2010; 38: 411–415.Google ScholarPubMed
3. Aly, AM, Red, DA, Jiwani, A, Desai, PH. Absent pulmonary valve syndrome with left hemitruncus. AJP Rep 2012; 2: 51–54.Google ScholarPubMed
4. Patel, RJ, Zakir, RM, Sethi, V, et al. Unrepaired tetralogy of Fallot with right hemitruncus in an adult: a rare case. Tex Heart Inst J 2007; 34: 250–251.Google Scholar
5. Dwived, SK, Vijay, SK, Chandra, S, Saran, RK. Left hemitruncus with tetralogy of Fallot and right aortic arch: rare survival beyond the first decade. Pediatric Cardol 2012; 33: 863–865.CrossRefGoogle Scholar
6. Presbitero, P, Pedretti, E, Orzan, F, et al. Absent pulmonary valve syndrome with associated anomalies of the pulmonary blood supply. Int J Cardiol 1984; 6: 587–598.CrossRefGoogle ScholarPubMed
Figure 0

Figure 1 Overriding of aorta with large subaortic ventricular septal defect (arrow) in long axis view, (a) absent pulmonary valve and left pulmonary artery (arrow) which is the sole artery arising from the right ventricle in short axis view in transthoracic echocardiography, (b) right pulmonary artery arising from ascending aorta in right parasternal view in transthoracic echocardiography (arrow), (c) • location of main pulmonary artery.

Figure 1

Figure 2 Anomalous origin of right pulmonary artery from left posterior side of ascending aorta. Aortic root injection at cardiac catheterization, (a) axial view of aorta and pulmonary artery in computed tomography (CT) angiography, showed left pulmonary artery is the sole artery originating from main pulmonary artery, (b) reconstructed coronal view of aorta and pulmonary artery in CT angiography revealed anomalous origin of right pulmonary artery from left posterolateral side of ascending aorta (arrow) which descend posteriorly towards right hillum, (c) *location of right pulmonary artery; LPA=left pulmonary artery; MPA=main pulmonary artery; RPA=right pulmonary artery.